Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study

Aleksander Kempny; Cristel Sørensen Hjortshøj; Hong Gu; Wei Li; Alexander R Opotowsky; Michael J Landzberg; Annette Schophuus Jensen; Lars Søndergaard; Mette-Elise Estensen; Ulf Thilén; Werner Budts; Barbara J Mulder; Ilja Blok; Lidia Tomkiewicz-Pająk; Kamil Szostek; Michele D'Alto; Giancarlo Scognamiglio; Katja Prokšelj; Gerhard-Paul Diller; Konstantinos Dimopoulos; Stephen J Wort; Michael A Gatzoulis

doi:10.1161/CIRCULATIONAHA.116.023033

Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study

Aleksander Kempny, Cristel Sørensen Hjortshøj, Hong Gu, Wei Li, Alexander R Opotowsky, Michael J Landzberg, Annette Schophuus Jensen, Lars Søndergaard, Mette-Elise Estensen, Ulf Thilén, Werner Budts, Barbara J Mulder, Ilja Blok, Lidia Tomkiewicz-Pająk, Kamil Szostek, Michele D'Alto, Giancarlo Scognamiglio, Katja Prokšelj, Gerhard-Paul Diller, Konstantinos DimopoulosStephen J Wort, Michael A Gatzoulis

Department of Clinical Medicine

54 Citations (Scopus)

Abstract

Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

Original language	English
Journal	Circulation
Volume	135
Issue number	15
Pages (from-to)	1432-1440
ISSN	0009-7322
DOIs	https://doi.org/10.1161/CIRCULATIONAHA.116.023033
Publication status	Published - 11 Apr 2017

Keywords

Adolescent
Adult
Aged
Aged, 80 and over
Biomarkers
Echocardiography
Eisenmenger Complex/diagnosis
Electrocardiography
Female
Follow-Up Studies
Humans
Male
Middle Aged
Oxygen Consumption
Phenotype
Prognosis
Proportional Hazards Models
Risk Assessment
Risk Factors
Severity of Illness Index
Walk Test
Young Adult

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1161/CIRCULATIONAHA.116.023033

http://hdl.handle.net/10044/1/44479Licence: CC BY-NC-ND

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Kempny, A., Hjortshøj, C. S., Gu, H., Li, W., Opotowsky, A. R., Landzberg, M. J., Jensen, A. S., Søndergaard, L., Estensen, M-E., Thilén, U., Budts, W., Mulder, B. J., Blok, I., Tomkiewicz-Pająk, L., Szostek, K., D'Alto, M., Scognamiglio, G., Prokšelj, K., Diller, G-P., ... Gatzoulis, M. A. (2017). Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study. Circulation, 135(15), 1432-1440. https://doi.org/10.1161/CIRCULATIONAHA.116.023033

Kempny, A, Hjortshøj, CS, Gu, H, Li, W, Opotowsky, AR, Landzberg, MJ, Jensen, AS, Søndergaard, L, Estensen, M-E, Thilén, U, Budts, W, Mulder, BJ, Blok, I, Tomkiewicz-Pająk, L, Szostek, K, D'Alto, M, Scognamiglio, G, Prokšelj, K, Diller, G-P, Dimopoulos, K, Wort, SJ & Gatzoulis, MA 2017, 'Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study', Circulation, vol. 135, no. 15, pp. 1432-1440. https://doi.org/10.1161/CIRCULATIONAHA.116.023033

@article{dde30a332fb44c7aafbdfb192da8f971,

title = "Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study",

abstract = "Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.",

keywords = "Adolescent, Adult, Aged, Aged, 80 and over, Biomarkers, Echocardiography, Eisenmenger Complex/diagnosis, Electrocardiography, Female, Follow-Up Studies, Humans, Male, Middle Aged, Oxygen Consumption, Phenotype, Prognosis, Proportional Hazards Models, Risk Assessment, Risk Factors, Severity of Illness Index, Walk Test, Young Adult",

author = "Aleksander Kempny and Hjortsh{\o}j, {Cristel S{\o}rensen} and Hong Gu and Wei Li and Opotowsky, {Alexander R} and Landzberg, {Michael J} and Jensen, {Annette Schophuus} and Lars S{\o}ndergaard and Mette-Elise Estensen and Ulf Thil{\'e}n and Werner Budts and Mulder, {Barbara J} and Ilja Blok and Lidia Tomkiewicz-Paj{\c a}k and Kamil Szostek and Michele D'Alto and Giancarlo Scognamiglio and Katja Prok{\v s}elj and Gerhard-Paul Diller and Konstantinos Dimopoulos and Wort, {Stephen J} and Gatzoulis, {Michael A}",

note = "{\textcopyright} 2016 American Heart Association, Inc.",

year = "2017",

month = apr,

day = "11",

doi = "10.1161/CIRCULATIONAHA.116.023033",

language = "English",

volume = "135",

pages = "1432--1440",

journal = "Circulation",

issn = "0009-7322",

publisher = "AHA/ASA",

number = "15",

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TY - JOUR

T1 - Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome

T2 - A Multicenter Study

AU - Kempny, Aleksander

AU - Hjortshøj, Cristel Sørensen

AU - Gu, Hong

AU - Li, Wei

AU - Opotowsky, Alexander R

AU - Landzberg, Michael J

AU - Jensen, Annette Schophuus

AU - Søndergaard, Lars

AU - Estensen, Mette-Elise

AU - Thilén, Ulf

AU - Budts, Werner

AU - Mulder, Barbara J

AU - Blok, Ilja

AU - Tomkiewicz-Pająk, Lidia

AU - Szostek, Kamil

AU - D'Alto, Michele

AU - Scognamiglio, Giancarlo

AU - Prokšelj, Katja

AU - Diller, Gerhard-Paul

AU - Dimopoulos, Konstantinos

AU - Wort, Stephen J

AU - Gatzoulis, Michael A

PY - 2017/4/11

Y1 - 2017/4/11

N2 - Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

AB - Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

KW - Adolescent

KW - Adult

KW - Aged

KW - Aged, 80 and over

KW - Biomarkers

KW - Echocardiography

KW - Eisenmenger Complex/diagnosis

KW - Electrocardiography

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Male

KW - Middle Aged

KW - Oxygen Consumption

KW - Phenotype

KW - Prognosis

KW - Proportional Hazards Models

KW - Risk Assessment

KW - Risk Factors

KW - Severity of Illness Index

KW - Walk Test

KW - Young Adult

U2 - 10.1161/CIRCULATIONAHA.116.023033

DO - 10.1161/CIRCULATIONAHA.116.023033

M3 - Journal article

C2 - 27979875

SN - 0009-7322

VL - 135

SP - 1432

EP - 1440

JO - Circulation

JF - Circulation

IS - 15

ER -

Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study

Abstract

Keywords

UN SDGs

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